J Korean Soc Pediatr Endocrinol.  2010 Dec;15(3):193-197.

Adrenocortical Carcinoma in a Patient with Congenital Adrenal Hyperplasia

• Cho MS
• Jin HY
• Choi JH
• Kim DY
• Lee JJ
• Yoo HW

Abstract

Adrenocortical carcinoma is a very rare condition in childhood. There are only a few reports about adrenocortical carcinomas associated with congenital adrenal hyperplasia. A two-month-old male baby presented at the local clinic with skin hyperpigmentation, dehydration, hyponatremia, and hyperkalemia. He was diagnosed with congenital adrenal hyperplasia and had been treated with hydrocortisone and 9alpha-fludrocortisone until 1 year of age. At age 5.8 years, he visited an outpatient clinic because of tall stature and secondary sexual characteristics, and hydrocortisone was administered. At age 16 years he was admitted for treatment of an adrenal tumor, incidentally detected during the evaluation of hematuria. Serum adrenocorticotrophic hormone and cortisol levels were 33.5 pg/mL and 5.2 microg/dL, respectively. 17alpha-hydroxyprogesterone level was 6.3 ng/mL and dehydroepiandrosterone sulfate level was 31.2 microg/dL. Abdominal ultrasonography and computed tomography revealed a solid mass of 7.7 x 5.6 x 6.2 cm in the left adrenal gland. It was totally removed by surgery, and the histopathology was compatible with adrenocortical carcinoma.

Keyword

Adrenocortical carcinoma; Adrenal hyperplasia, congenital

MeSH Terms

Adrenal Glands
Adrenal Hyperplasia, Congenital
Adrenocortical Carcinoma
Adrenocorticotropic Hormone
Ambulatory Care Facilities
Dehydration
Dehydroepiandrosterone Sulfate
Hematuria
Humans
Hydrocortisone
Hyperkalemia
Hyperpigmentation
Hyponatremia
Male
Skin
Adrenocorticotropic Hormone
Dehydroepiandrosterone Sulfate
Hydrocortisone